Palliative care: Not just end of life care

My daughter Esmé is a four-and-one-half-year-old girl who loves listening to “The Muppets Movie” soundtrack, laughing at silly jokes and reading books. Esmé has been a patient at Boston Children’s Hospital since she was 8 months old, when she started seeing the Aerodigestive Clinic for chronic aspiration. She is followed by a number of specialists to treat her epilepsy, low tone, kidney and heart abnormalities, developmental delay, poor growth, lack of verbal communication, among other things. Esmé is presumed to have a genetic disorder, but, so far, she does not have a clear diagnosis.

Last week I had an appointment that I was both really looking forward to and dreading, simultaneously. The appointment was with Pediatric Advanced Care Team (PACT) at Boston Children’s. They are the group who does palliative care there.

Now, I know that most people hear “palliative care” and think very specific things about end of life care.

I did, certainly.

And, frankly, my associations with palliative care were enough to make me not want to have the meeting with them in the first place.

However, we have reached that place in Esmé’s care where, while she much more stable than ever, there are some roadblocks that affect her (and our family’s) quality of life. Several of my mom friends who also have children with similar needs suggested that it might be time to talk to palliative care.

Wow, right? Obviously Ez has a serious illness. And, I’m sure this will surprise all of you, but there is stress (oh, God the stress …). While palliative care can be a method used in conjunction with end of life concerns, it is fundamentally focused on living–living happily and comfortably despite on-going serious health challenges. And, because we are in this for the longterm, it seems like improving Esmé’s quality of life–and the quality of life of our family unit–is sort of a no-brainer…

So, I asked our neurologist to help us make an appointment … despite the little nagging concerns I had that palliative care might not be for us.

I didn’t know what to expect from PACT–but I made a short and actionable list of the two things I thought they could help with immediately: Ezzy’s wanky sleep and her unexplained (kidney?) pain. There is a longer more hopeful list of things to get help with down the line, which includes school planning with our district, visits with the Muppets, flying girafficorns (yes that is a thing) as a service animal and adaptive mobility equipment…but I planned to let them start with the easy stuff–you know, before they figure out the impossible standard I will hold them to.

I have to say that the meeting exceeded my expectations. The appointment really was a brainstorming and planning session. Of course there was a good deal of getting to know each other, of sharing Ezzy’s history and so on. But the team seemed to quickly get our approach to Ezzy, which involves a minimalist approach to medications (all things considered), an awkward sense of humor, a willingness to try anything to give her an experience that might be meaningful for her.

We talked about Esmé’s sleep at length. I don’t think I have explained in much detail here what Ezzy’s evenings and overnights can be like. But Ez is still rocked to sleep because she really cannot or does not settle on her own, and she cannot be left alone in her crib for any period of time especially if she is unhappy because of her risks of breath-holding and injury. For awhile this wasn’t a problem. I’d rock her for 45 minutes or so and then set her down in her crib fully asleep. It was a nicely contained snuggle session.

However, over the last year and a half she has begun to have trouble falling asleep, maintaining sleep in my arms and staying asleep when I transfer her. This has meant that she has multiple-hour sessions of full holding and rocking—during which time she can be resting or “talking” which is nice…but often she is crying, flailing, jolting awake, and, sometimes, hitting, headbutting and breath-holding. We’ve started taking 45-60 minute shifts with her—which is a frustration and back-saver—but it has meant that our entire evening, every night is consumed with bedtime. And there have been no signs of it improving on its own.

Often Ez is up again in the middle of the night—either having fun and being silly or crying or twitching or retching or some combination. Thankfully we have regular night nursing to help with the middle of the night—because Ez cannot be left to play quietly in her crib, and bringing her in bed with us is no longer a soothing treat, but is often infuriating for her for some reason.

So, the PACT helped talk through all of that, asked some questions and helped make a plan to try out … beginning with waking Esmé up every morning at the same time, no matter how little sleep she had the night before. And they also suggested only starting her bedtime routine at 9 p.m., keeping it very regular and predictable—and minimizing the choices she gets to make at this time (one book or two—instead of another book? and another?).

As they were talking to me it suddenly clicked: Oh this is sort of an age-appropriate typical kid problem that has been masked by her developmental delay and medical fragility. We have been so focused on supporting her choice-making, that we never considered that having open-ended choices before bedtime might excite her more than sooth her. And I have been so focused on her having the sleep she needs, that I’m afraid to wake her after a night where she’s only slept for five hours.

So, we’ve been trying the new schedule. It isn’t a silver bullet, but it is helping reduce everyone’s stress, I think. And I feel really good about that.

The team will also be meeting with us and Ezzy’s urologist in a week so that we can trouble-shoot the kidney stuff together. Honestly it is such a relief to have a group of people I can talk to about the larger picture of Esmé’s care. I recognize that there is a limit to the support they will be able to provide, of course (fingers crossed on the girafficorn, though), but it feels really good to have the extra support there.